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Rsph9 is critical for ciliary radial spoke assembly and central pair microtubule stability
Biology of the Cell ( IF 2.4 ) Pub Date : 2018-11-16 , DOI: 10.1111/boc.201800060
Lei Zhu 1, 2 , Hao Liu 1, 2 , Yawen Chen 1, 2 , Xiumin Yan 1, 2 , Xueliang Zhu 1, 2
Affiliation  

In the “9+2”‐type motile cilia, radial spokes (RSs) protruded from the nine peripheral microtubule doublets surround and interact with the central pair (CP) apparatus to regulate ciliary beat. RSPH9 is the human homologue of the essential protozoan RS head protein Rsp9. Its mutations in human primary ciliary dyskinesia patients, however, cause CP loss in a small portion of airway cilia without affecting the ciliary localization of other head proteins.

中文翻译:

Rsph9 对睫状径向辐条组装和中央对微管稳定性至关重要

在“9+2”型运动纤毛中,从九个外围微管双联体突出的径向辐条(RS)围绕中央对(CP)装置并与之相互作用以调节纤毛搏动。RSPH9 是必需原生动物 RS 头部蛋白 Rsp9 的人类同源物。然而,它在人类原发性纤毛运动障碍患者中的​​突变会导致一小部分气道纤毛的 CP 丢失,而不会影响其他头部蛋白的纤毛定位。
更新日期:2018-11-16
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